Shaken baby syndrome.

PURPOSE: To examine the comprehensive ophthalmologic experience with the shaken baby syndrome at one medical center, including clinical findings, autopsy findings, and the outcome of survivors. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: One hundred twenty-three children admitted from January 1987 through December 1998 for subdural hematomas of the brain secondary to abuse were included. METHODS: Clinical features of eye examinations of the patients during their admission and after discharge and histopathologic observations for patients who died were retrieved from medical records and statistically analyzed. MAIN OUTCOME MEASURES: Visual response and pupillary response on initial examination, fundus findings, final vision, neurologic outcome of survivors, and death. RESULTS: Ninety percent of the patients had ophthalmologic assessments. Retinal hemorrhages were detected in 83% of the examined children. The retinal hemorrhages were bilateral in 85% of affected children and varied in type and location. Nonophthalmologists missed the hemorrhages in 29% of affected patients. Poor visual response, poor pupillary response, and retinal hemorrhage correlated strongly with the demise of the child. One child who died had pigmented retinal scars from previous abuse, a condition not previously observed histopathologically to our knowledge. One fifth of the survivors had poor vision, largely the result of cerebral visual impairment. Severe neurologic impairment correlated highly with loss of vision. CONCLUSIONS: Shaken baby syndrome causes devastating injury to the brain and thus to vision. Retinal hemorrhages are extremely common, but vision loss is most often the result of brain injury. The patient's visual reaction and pupillary response on presentation showed a high correlation with survival. Good initial visual reaction was highly correlated with good final vision and neurologic outcome. According to the literature, when retinal hemorrhages are found in young children, the likelihood that abuse occurred is very high. Nonophthalmologists' difficulty in detecting retinal hemorrhages may be an important limiting factor in identifying shaken babies so they can be protected from further abuse.

Effect of prior orbital decompression on outcome of strabismus surgery in patients with thyroid ophthalmopathy.

PURPOSE: To compare strabismus surgery outcomes of patients who have had prior orbital decompression for thyroid ophthalmopathy with those of patients who have not had decompression. METHODS: The records of all patients operated on by the author for strabismus related to thyroid ophthalmopathy were retrospectively reviewed. RESULTS: Fifty patients were included in this study. Seventeen patients had previously undergone orbital decompression, and 33 patients had not. Seventy-six percent of patients who had had orbital decompression had a good or excellent outcome compared with 91% of those who had not had orbital decompression. Patients in the orbital decompression group had an average of 1.4 operations compared with 1.2 in the no-decompression group. The average numbers of muscles operated on were 3.1 in the decompression group and 1.9 in the no-decompression group. Patients who had been decompressed were more than 4 times as likely to require surgery for both a horizontal and vertical deviation than patients who had not been decompressed. CONCLUSIONS: Patients with thyroid ophthalmopathy who have had orbital decompression have a lower success rate of surgery for strabismus, more frequently need correction for both horizontal and vertical deviations, and have more muscles operated on than patients who have not had orbital decompression. The need for orbital decompression in patients with Graves' disease is reflective of a worse degree of orbitopathy. In addition, ocular changes from decompression surgery may interfere with a successful result from strabismus surgery.

Ocular findings after bone marrow transplantation in a pediatric population.

PURPOSE: With the increasing use of bone marrow transplantation (BMT) in children to treat diseases of the hematopoietic system, ocular complications have been recognized in greater numbers. The authors performed a retrospective study to determine the prevalence and types of ocular abnormalities in a pediatric population following BMT. DESIGN: Retrospective noncomparative study. PARTICIPANTS: One hundred and four consecutive pediatric patients with aplastic anemia and various hematologic malignancies who required BMT. INTERVENTION: Bone marrow transplantation. MAIN OUTCOME MEASURES: Visual acuity and slit-lamp biomicroscopic and funduscopic examinations. RESULTS: Ocular changes developed in 51% of patients. The most frequent findings included dry eye syndrome (12.5%), cataract (23.0%), and posterior segment complications (13.5%). A final visual acuity of 20/40 or better was achieved in 95.7% of eyes. CONCLUSIONS: Pediatric patients can develop severe and potentially vision-threatening complications following BMT. Despite the high incidence of anterior and posterior segment abnormalities, ocular symptoms were generally mild and the majority of patients retained excellent visual function. Factors associated with ocular complications included the underlying disease, total body irradiation, systemic chemotherapy, graft-versus-host disease, and immunosuppression. Familiarity with these risk factors and potential complications in pediatric patients is important for successful treatment.

Visual outcomes following lensectomy and vitrectomy for combined anterior and posterior persistent hyperplastic primary vitreous.

OBJECTIVE: To determine the visual outcome after surgery for persistent hyperplastic primary vitreous using modern vitreoretinal techniques. DESIGN: Retrospective medical record review during a 5-year period (June 1992 to June 1997). Information recorded for each patient included age, medical history, sex, results of preoperative ocular examination, age at diagnosis, procedure performed, intraoperative and postoperative complications, location and number of sclerotomy sites, type of aphakic rehabilitation, amblyopic therapy given, final visual acuity, and length of follow-up. RESULTS: Fourteen patients who underwent surgical management of combined anterior and posterior persistent hyperplastic primary vitreous were identified. Eleven patients underwent aphakic rehabilitation and aggressive amblyopic therapy consisting of occlusive therapy for several waking hours each day. One additional older patient received aphakic rehabilitation only. Ten eyes (71%) achieved a visual acuity of 20/300 or better, and 8 (57%) obtained a final visual acuity of 20/100 or better. Average length of follow-up was 22 months (range, 4-57 months). Nine patients were fitted with an aphakic soft contact lens, 2 older patients had a posterior chamber intraocular lens placed at the time of vitrectomy, and 1 patient wore aphakic spectacles. CONCLUSIONS: With modern vitreoretinal techniques, aphakic rehabilitation, and aggressive amblyopic therapy, useful vision can be obtained in the majority of patients with combined anterior and posterior persistent hyperplastic primary vitreous.

Chorioretinal colobomas in a pediatric population.

OBJECTIVE: The purpose of the study was to determine the prevalence of retinal detachment and associated anomalies in pediatric patients with chorioretinal colobomas. DESIGN: Observational case series. PARTICIPANTS: Forty-eight patients (86 eyes) of patients with chorioretinal colobomas followed in two academic pediatric eye clinics were reviewed. MAIN OUTCOME MEASURES: The frequency of associated systemic abnormalities and the prevalence of retinal or choroidal detachment during the follow-up period. RESULTS: Forty-eight patients (86 eyes) were identified; ocular involvement ranged from small colobomas with isolated chorioretinal involvement to large colobomas affecting the iris, choroid, retina, and optic nerve. The mean ages at initial and most recent examinations were 27 and 100 months, respectively. Follow-up averaged 6 years, 1 month. Six retinal detachments were found in four patients. Patient ages at the time of detection of retinal detachment were 5 months, 29 months, 10 years, and 15 years. A choroidal detachment was found in a child at age 9 years. The combined prevalence of retinal or choroidal detachment was 10.4% of patients and 8.1% of affected eyes. Thirteen eyes were microphthalmic, and six had microcornea. Eighteen patients (38%) had other systemic abnormalities. CONCLUSIONS: The 8.1% prevalence of retinal or choroidal detachment among eyes in our series was much smaller than previously cited estimates of 23% to 43%. The higher figures probably reflect a referral bias toward patients with vitreoretinal problems and an older patient population. Because of the acknowledged difficulty of managing retinal detachments in patients with chorioretinal colobomas, emphasis should be directed toward early detection and possible prophylactic therapy. The high proportion of patients who have associated systemic abnormalities indicates the need for thorough evaluation of these children by other pediatric specialists.

Initial versus subsequent postoperative motor alignment in intermittent exotropia.

BACKGROUND: Although initial overcorrection is believed to be important after bilateral lateral rectus muscle recessions for intermittent exotropia, not all patients with desirable amounts of initial overcorrection have good final outcomes. The purpose of this study is to evaluate the relationship between initial postoperative and subsequent postoperative motor outcomes in a group of patients operated on for intermittent exotropia. METHODS: All patients on whom I performed bilateral lateral rectus muscle recessions as the initial surgical procedure for intermittent exotropia and who had at least 6 months of postoperative follow-up were included in this study. RESULTS: Of the 60 patients in this study, 38 (63%) had good outcomes (< or = 10 PD exophoria or < or = 5 PD esophoria), 15 (25%) had undercorrection (> 10 PD exodeviation), and seven (12%) had overcorrection (> 5 PD esodeviation). The chance of a good outcome was highest with initial postoperative alignment between orthotropia and 9 PD of esotropia, but 22% of patients with alignment in this range after the operation ended up overcorrected or undercorrected. Most patients had an exotropic drift after the operation, but seven patients had a drift in an esotropic direction. CONCLUSIONS: Although an initial alignment within the range of orthotropia to 9 PD of esotropia during the first few days after the operation is desirable for patients with intermittent exotropia, alignment within this range does not guarantee a good final outcome, nor does alignment outside this range guarantee a bad outcome. Little predictability exists with respect to the amount and occasionally even the direction of postoperative drift. This unpredictability may in part reflect the artifactual nature of the initial postoperative measurement.

Subgaleal hematoma with delayed proptosis and corneal ulceration.

We report the delayed occurrence in a child of proptosis caused by a subgaleal hematoma. A corneal ulcer resulted from the proptosis, which developed 9 days after mild head trauma. Evaluation revealed a heterozygous factor VII deficiency. The coagulopathy and, more likely, the use of the nonsteroidal antiinflammatory drug ibuprofen may have contributed to the extension of the hematoma into the orbital space. Careful follow-up of patients with subgaleal hematomas is necessary because the development of proptosis may be delayed.

Odonto-onycho-dermal dysplasia.

Odonto-onycho-dermal dysplasia is an ectodermal dysplasia (ED) described once previously in two families who exhibited atrophic malar patches, sparse hair, conical teeth, dystrophic nails and hyperkeratosis of the palms and soles. We describe a boy who developed a blistering malar rash soon after birth. When examined at 11 months, and then at 27 months of age, he had persistent atrophic malar plaques that reddened with heat. He also showed nail dystrophy, sparse hair, lingual concavity of the incisors, a bifid maxillary incisor, a five-cusped molar, and hyperhidrosis of the palms and soles. In addition he had chronic tearing, photophobia, blepharitis, and a mild keratitis. After reviewing EDs with atrophic or scar-like skin changes, we believe this child most resembles the patients with odonto-onycho-dermal dysplasia, although his eye findings are unique.

Trabeculectomy for traumatic hyphema with increased intraocular pressure.

We reviewed the medical records of 11 consecutive patients who underwent trabeculectomy with anterior chamber washout and peripheral iridectomy as the primary surgical treatment for traumatic hyphema that was unresponsive to medical management. The mean intraocular pressure before surgery was 48 mm Hg. In ten of the patients the intraocular pressure was lowered to 21 mm Hg or lower after surgery and remained below that level up to the most recent follow-up visit, which ranged from eight to 97 months. One patient required a topical beta-blocker and oral acetazolamide to lower pressure to this level after surgery. Eight patients had visual acuity of 20/60 or better at last follow-up. Corneal blood staining occurred in eight patients. Compared with other techniques for surgical management of traumatic hyphema, trabeculectomy provides a means to keep intraocular pressure lowered while the remaining blood is clearing from the anterior chamber. Trabeculectomy with anterior chamber washout and peripheral iridectomy appears to be a safe and reliable procedure in the management of traumatic hyphemas in which medical management fails to control intraocular pressure.

Pseudophakia for traumatic cataracts in children.

PURPOSE: The purpose of this prospective study is to evaluate the postoperative visual acuity, refractive error, intraocular pressure, and status of the posterior capsule in children with traumatic cataracts who undergo extracapsular cataract extraction and insertion of a posterior chamber lens. METHODS: Extracapsular cataract extraction and primary endocapsular fixation of a posterior chamber lens implant were performed in eight children (age range, 4-17 years) with unilateral traumatic cataracts. RESULTS: There were no intraoperative complications, and seven of eight eyes achieved 20/40 or greater spectacle visual acuity during an average follow-up interval of 10 months (range, 5-20 months). The average postoperative spherical equivalent refractive error was +0.33 diopter (D) (range, -2.25 to +2.12 D); the average postoperative anisometropia was approximately 1 D (range, 0-2.25 D). In one patient, a coagulase-negative staphylococcal endophthalmitis developed 10 days after surgery. In three eyes that had opacified posterior capsules, YAG laser capsulotomy was performed. CONCLUSIONS: These preliminary results suggest that intraocular lens (IOL) implantation may be a safe and effective method of optical correction for children with traumatic cataracts.

Bilateral retinal embolization associated with intralesional corticosteroid injection for capillary hemangioma of infancy.

A 2-month-old female infant underwent intralesional corticosteroid injection for a capillary hemangioma that was causing amblyopia of the right eye from ptosis, globe displacement, and astigmatism. Forty-eight hours after the injection, the infant's parents noted that she was visually inattentive. On examination, she could not fixate or follow with either eye, and an afferent pupillary defect was present in the left eye. Ophthalmoscopy showed scattered areas of intraretinal hemorrhage in the right eye and extensive preretinal and intraretinal hemorrhages in the left eye. Ten months after injection, the hemangioma had greatly regressed, and visual acuity in the right eye was felt to be normal. The left eye had unsteady fixation with a persistent afferent pupillary defect and macular scarring. Retrograde flow of the corticosteroid suspension through the hemangioma's feeder vessels, probably originating from both ophthalmic arteries, is the most likely explanation for this complication.

Affected females in X-linked congenital stationary night blindness.

Most heterozygous (carrier) females in families with X-linked congenital stationary night blindness are asymptomatic. Several anecdotal cases of manifesting females in X-linked congenital stationary night blindness have been reported, but few clinical details are available. The authors report clinical, electroretinographic, and dark adaptation studies of four affected females from a five-generation family with X-linked congenital stationary night blindness. Each of the manifesting females was the daughter of a different, asymptomatic, carrier mother. None of the 14 daughters of the 9 affected males showed signs or symptoms of congenital stationary night blindness. Uneven X-chromosomal lyonization is the most likely reason for these females manifesting this X-linked disorder.